FUCA1 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	WB
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	The immunogen for Anti-FUCA1 Antibody: synthetic peptide directed towards the N terminal of human FUCA1. Synthetic peptide located within the following region: PSPVSWNWNSKDVGPHRDLVGELGTALRKRNIRYGLYHSLLEWFHPLYLL
Isotype	IgG
Formulation	Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. Note that this product is shipped as lyophilized powder to China customers.
Purification	Affinity Purified
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Predicted Protein Size	54 kDa
Gene Name	fucosidase, alpha-L- 1, tissue
Database Link	NP_000138 Entrez Gene 2517 Human UniProt ID P04066
Background	Alpha-L-fucosidase (EC 3.2.1.51) is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. At least 2 separate polymorphic alpha-L-fucosidases are recognized in man: that in tissues, FUCA1, which is deficient in fucosidosis, and that in plasma, FUCA2. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form; coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form; and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form. [supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
Synonyms	FUCA
Note	Immunogen Sequence Homology: Pig: 100%; Rat: 100%; Human: 100%; Mouse: 100%; Bovine: 100%; Dog: 93%; Horse: 93%; Guinea pig: 93%; Rabbit: 79%
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Lysosome, Other glycan degradation

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